Hematology Practice Questions

Q: A couple with a family history of beta-thalassemia major in a distant relative has come for counseling. The husband has HbA2 of 4.8% and the wife has HbA2 of 2.3%. What is the risk of them having a child with beta-thalassemia major?

0%. ### Explanation **1. Understanding the Correct Answer (D: 0%)** To have a child with **Beta-Thalassemia Major**, both parents must be carriers (**Beta-Thalassemia Trait/Minor**). * **The Husband:** His HbA2 is **4.8%**. In clinical practice, an HbA2 level **>3.5%** is the diagnostic hallmark of Beta-Thalassemia Trait. Thus, the husband is a carrier. * **The Wife:** Her HbA2 is **2.3%**. The normal range for HbA2 is **1.5% to 3.5%**. Since her value is within the normal range, she is not a carrier of the beta-thalassemia gene. Beta-thalassemia is an **autosomal recessive** disorder. For a child to manifest the "Major" form ($β^0/β^0$ or $β^+/β^+$), they must inherit one defective gene from each parent. Since the wife is hematologically normal, she will always pass on a normal beta-globin gene. Therefore, the risk of having a child with Thalassemia Major is **0%**. (The risk of having a child with Thalassemia Trait is 50%). **2. Why Other Options are Incorrect** * **A (50%) & B (25%):** These probabilities would only apply if both parents were carriers. If both were carriers, the risk for Major would be 25% and for Trait would be 50%. * **C (5%):** This is a distractor with no genetic basis in Mendelian inheritance for this condition. **3. NEET-PG High-Yield Pearls** * **Gold Standard Diagnosis:** Hb electrophoresis or HPLC (High-Performance Liquid Chromatography). * **HbA2 Levels:** * Normal: 1.5–3.5% * β-Thalassemia Trait: >3.5% (usually 4–8%) * Iron Deficiency Anemia: Can falsely **lower** HbA2 levels, potentially masking a Thalassemia trait. * **Mentzer Index:** MCV/RBC count. If ** 13**, it suggests Iron Deficiency Anemia.

Q: Cold agglutinins are seen in which of the following conditions?

Influenza. **Explanation:** Cold agglutinins are autoantibodies (usually **IgM**) that bind to the 'I' or 'i' antigens on the surface of red blood cells at low temperatures (typically <30°C), leading to agglutination and extravascular hemolysis [1]. **1. Why Influenza is correct:** Cold agglutinin disease is frequently secondary to specific infections. The most common triggers are ***Mycoplasma pneumoniae*** (associated with anti-I antibodies) and viral infections like **Infectious Mononucleosis (EBV)**, **Cytomegalovirus (CMV)**, and **Influenza** [2]. These pathogens can trigger a transient, polyclonal IgM response that cross-reacts with RBC antigens [2]. **2. Why the other options are incorrect:** * **Polyarteritis nodosa (PAN):** This is a systemic necrotizing vasculitis of medium-sized arteries. It is classically associated with Hepatitis B, but not with cold agglutinins. * **Multiple myeloma:** This is a plasma cell dyscrasia characterized by IgG or IgA monoclonal spikes. While it can cause anemia of chronic disease or hyperviscosity, it is not a typical cause of cold agglutinins. * **Systemic lupus erythematosus (SLE):** SLE is the classic cause of **Warm Autoimmune Hemolytic Anemia (WAIHA)**, mediated by **IgG** antibodies that react at body temperature (37°C) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Warm AIHA:** IgG; associated with SLE, CLL, and drugs (α-methyldopa) [1]. * **Cold AIHA:** IgM; associated with *Mycoplasma*, EBV, and Lymphoma [2]. * **Blood Smear:** Look for **RBC clumps/agglutination** (unlike Rouleaux, which is linear) [2]. * **Direct Coombs Test:** In Cold AIHA, the test is positive for **C3d** (complement) but negative for IgG [1].

Q: Regarding multiple myeloma, which of the following statements is wrong?

Multiple hot spots on bone scan.. In Multiple Myeloma (MM), the primary mechanism of bone destruction is **osteoclastic activation** coupled with the **suppression of osteoblasts**. Bone scans (Technetium-99m MDP) rely on osteoblastic activity (new bone formation) to show "hot spots." Because MM lesions are purely lytic with minimal to no osteoblastic response, bone scans are notoriously insensitive and often appear "cold" or normal despite extensive bone involvement. Therefore, statement C is incorrect. **Analysis of other options:** * **Option A:** MM is by definition a malignant proliferation of monoclonal plasma cells in the bone marrow, producing an M-protein spike [1]. * **Option B:** The classic radiographic hallmark of MM is "punched-out" lytic lesions, most visible on a skeletal survey of the skull, spine, and long bones [1]. * **Option C (Correct Answer):** As explained, bone scans are typically negative; **MRI or PET-CT** are the preferred advanced imaging modalities. * **Option D:** Melphalan (an alkylating agent) remains a cornerstone of treatment, especially in transplant-ineligible patients (often combined with Prednisone and Bortezomib). **High-Yield Clinical Pearls for NEET-PG:** * **CRAB Criteria:** Calcium (elevated), Renal insufficiency, Anemia, and Bone lesions. * **Diagnosis:** Bone marrow plasma cells ≥10% or biopsy-proven plasmacytoma PLUS myeloma-defining events [1]. * **Bence-Jones Proteins:** These are free light chains in the urine; they are **not** detected by standard dipsticks (which detect albumin). * **M-Spike:** Usually IgG (most common) or IgA. * **Peripheral Smear:** Characterized by **Rouleaux formation** due to increased serum proteins [1].

Q: A 25-year-old female with a known history of sickle cell anemia presents with bony pain, dyspnea, fever, and cough. On examination, her heart rate is 110/min, blood pressure is 140/88 mmHg, and SaO2 is 85%. Chest X-ray shows bilateral diffuse alveolar infiltrates. Her mother reports four similar episodes in the past year. Which of the following statements regarding her condition is false?

She should receive daily sildenafil.. The clinical presentation of bony pain, fever, dyspnea, and hypoxia (SaO2 85%) with new pulmonary infiltrates on chest X-ray in a patient with sickle cell disease (SCD) is diagnostic of **Acute Chest Syndrome (ACS)**. [1] **Why Option C is the Correct (False) Statement:** While pulmonary hypertension is a common complication of SCD, the **Walk-PHaSST trial** demonstrated that **Sildenafil** (a PDE-5 inhibitor) is associated with an **increased frequency of vaso-occlusive crises** (painful crises) in SCD patients. Therefore, it is not recommended as standard daily therapy for SCD-related pulmonary complications and is the false statement in this context. **Analysis of Other Options:** * **Option A (True):** Hydroxyurea increases fetal hemoglobin (HbF) levels, which inhibits the polymerization of HbS. [2] It is the standard of care for patients with recurrent ACS (≥2 episodes) or frequent painful crises to reduce the frequency of these events. * **Option B (True):** The patient meets the diagnostic criteria for ACS: a new pulmonary infiltrate involving at least one complete lung segment, plus systemic symptoms (fever, chest pain, or respiratory distress). * **Option D (True):** Management of ACS involves judicious fluid administration and blood transfusions (simple or exchange) to improve oxygenation. [2] The goal is to maintain a Hematocrit >30% and reduce HbS levels to <30% in severe cases. **High-Yield Clinical Pearls for NEET-PG:** * **ACS Triggers:** Often precipitated by pulmonary infarction (fat embolism from bone marrow) [1] or infections (*Chlamydia, Mycoplasma*). * **Treatment Triad for ACS:** Antibiotics (including a Macrolide), Oxygen, and Transfusion. * **Hydroxyurea Side Effect:** Myelosuppression (monitor CBC regularly). It is also teratogenic. * **Most common cause of death** in adult SCD patients is Acute Chest Syndrome.

Q: What is true about smoldering multiple myeloma?

Presence of monoclonal gammopathy. Smoldering Multiple Myeloma (SMM) is an intermediate clinical stage between Monoclonal Gammopathy of Undetermined Significance (MGUS) and symptomatic Multiple Myeloma. **Why Option A is Correct:** By definition, SMM requires the presence of a **monoclonal (M) protein** (serum IgG or IgA ≥30 g/L) or urinary monoclonal protein (≥500 mg/24 h) [1]. Therefore, the presence of monoclonal gammopathy is a fundamental diagnostic requirement. **Why the Other Options are Incorrect:** * **Options B & C:** The hallmark of SMM is the **absence of end-organ damage**, commonly remembered by the acronym **CRAB** (Calcium elevation, Renal insufficiency, Anemia, and Bone lesions). If lytic bone lesions or hypercalcemia are present, the diagnosis upgrades to active Multiple Myeloma [1]. * **Option D:** While SMM does require bone marrow plasma cells (BMPC) to be between **10% and 60%**, this option is technically incomplete/vague compared to the definitive presence of monoclonal gammopathy. Note: If BMPC >60%, it is now classified as active Multiple Myeloma even without CRAB features (SLiM-CRAB criteria) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Diagnostic Criteria for SMM:** Serum M-protein ≥30 g/L OR BMPC 10–60% AND absence of myeloma-defining events (CRAB features) [1]. * **Risk of Progression:** SMM has a progression rate to overt myeloma of approximately 10% per year for the first five years. * **SLiM Criteria (Biomarkers of Malignancy):** Even without CRAB, it is Multiple Myeloma if: 1. **S**ixty percent (60%) or more BMPC. 2. **Li**ght chain ratio (involved:uninvolved) ≥100. 3. **M**RI showing >1 focal bone lesion.

Q: A young female presents with severe abdominal pain, constipation, irrelevant talking, a BP of 160/110 mmHg, heart rate of 120/min, fever, and a positive Watson-Schwartz test in urine. Which of the following drugs should not be used in this condition?

All of the above. ### Explanation **Diagnosis: Acute Intermittent Porphyria (AIP)** The clinical presentation—**abdominal pain, constipation, neuropsychiatric symptoms** (irrelevant talking), and **autonomic instability** (hypertension, tachycardia, fever)—is the classic "tetrad" of AIP [1]. The **Watson-Schwartz test** confirms the diagnosis by detecting elevated levels of porphobilinogen (PBG) in the urine. **The Underlying Concept: Enzyme Induction** AIP is caused by a deficiency in the enzyme *hydroxymethylbilane synthase* [1]. The condition is exacerbated by any factor that induces **Cytochrome P450 (CYP450)** enzymes. When these enzymes are induced, the demand for "Heme" increases, which upregulates **ALA Synthase** (the rate-limiting enzyme). This leads to a toxic accumulation of porphyrin precursors (ALA and PBG), triggering an acute attack. **Why "All of the Above" is Correct:** All three substances listed are potent **porphyrinogenic agents**: * **Sodium Valproate:** An anticonvulsant that induces hepatic enzymes and is strictly contraindicated in porphyria [1]. * **Alcohol:** A common trigger that induces ALA synthase and interferes with the heme biosynthetic pathway [1]. * **Griseofulvin:** An antifungal drug known to be a potent inducer of CYP450 enzymes, frequently cited in exams as a trigger for AIP. **Clinical Pearls for NEET-PG:** * **The 5 P’s of AIP:** **P**ainful abdomen, **P**olyneuropathy, **P**sychiatric disturbances, **P**ink urine (on standing), and **P**recipitated by drugs [1]. * **Safe Drugs:** If a patient with AIP needs treatment, safe options include **Aspirin, Paracetamol, Morphine, and Gabapentin.** * **Management:** The treatment of choice for an acute attack is **Intravenous Hematin** (which provides negative feedback to ALA synthase) and **High-dose Glucose** (which inhibits ALA synthase transcription).

Question 1

A couple with a family history of beta-thalassemia major in a distant relative has come for counseling. The husband has HbA2 of 4.8% and the wife has HbA2 of 2.3%. What is the risk of them having a child with beta-thalassemia major?

Accepted Answer

0%

Answer

50%

Answer

25%

Answer

5%

Question 2

Cold agglutinins are seen in which of the following conditions?

Accepted Answer

Influenza

Answer

Polyarteritis nodosa (PAN)

Answer

Multiple myeloma

Answer

Systemic lupus erythematosus (SLE)

Question 3

The parents of a 13-month-old boy are contacted by a local social agency because the day care reported them for suspected child abuse based on 'too many bruises.' The parents deny the abuse and promptly arrange to have their child seen by a medical specialist. The child has multiple large bruises on his legs, anus, and buttocks. No skin abrasions and no 'pattern marks' are seen. PTT is prolonged and PT and bleeding time are normal. Which of the following is the most probable diagnosis?

Accepted Answer

Von Willebrand disease

Answer

Acute myelogenous leukemia

Answer

Disseminated intravascular coagulation

Answer

Vitamin K deficiency

Question 4

Which of the following conditions is associated with raised levels of fetal hemoglobin (HbF)?

Accepted Answer

Juvenile chronic myeloid leukemia (CML)

Answer

Hereditary spherocytosis

Answer

Congenital red cell aplasia

Answer

Myasthenia gravis

Question 5

Decreased osmotic fragility is seen in which of the following conditions?

Accepted Answer

Thalassemia

Answer

Hereditary spherocytosis

Answer

Sickle cell disease

Answer

Autoimmune hemolytic anemia

Question 6

Regarding multiple myeloma, which of the following statements is wrong?

Accepted Answer

Multiple hot spots on bone scan.

Answer

Malignant neoplasm derived from plasma cells.

Answer

Multiple punched-out lesions on radiograms of the skull.

Answer

Melphalan is used in treatment.

Question 7

Compared to a hemolytic crisis, an aplastic crisis in sickle cell anemia leads to which of the following?

Accepted Answer

Decrease in reticulocytes

Answer

Increased blood volume

Answer

Total leukocyte count decreased

Answer

Same findings

Question 8

A 25-year-old female with a known history of sickle cell anemia presents with bony pain, dyspnea, fever, and cough. On examination, her heart rate is 110/min, blood pressure is 140/88 mmHg, and SaO2 is 85%. Chest X-ray shows bilateral diffuse alveolar infiltrates. Her mother reports four similar episodes in the past year. Which of the following statements regarding her condition is false?

Accepted Answer

She should receive daily sildenafil.

Answer

Chronic therapy with oral hydroxyurea should be considered.

Answer

She is experiencing acute chest syndrome related to sickle cell disease.

Answer

Hematocrit should be maintained at greater than 30%.

Question 9

What is true about smoldering multiple myeloma?

Accepted Answer

Presence of monoclonal gammopathy

Answer

Presence of lytic bone lesions

Answer

Presence of hypercalcemia

Answer

Bone marrow plasma cells > 10%

Question 10

A young female presents with severe abdominal pain, constipation, irrelevant talking, a BP of 160/110 mmHg, heart rate of 120/min, fever, and a positive Watson-Schwartz test in urine. Which of the following drugs should not be used in this condition?

Accepted Answer

All of the above

Answer

Sodium Valproate

Answer

Alcohol

Answer

Griseofulvin

Hematology — MCQs

Hematology — MCQs

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